Multimedia Encyclopedia

Eisenmenger syndrome

Eisenmenger syndrome is a condition that affects blood flow from the heart to the lungs in some babies who have structural problems of the heart.

Causes

Eisenmenger syndrome is caused by a defect in the heart. Most often, babies with this condition are born with a hole between the two pumping chambers -- the left and right ventricles -- of the heart (ventricular septal defect). The hole allows blood that has already picked up oxygen from the lungs to flow back into the lungs, instead of going out to the rest of the body.

Ventricular septal defect

Ventricular septal defect describes one or more holes in the wall that separates the right and left ventricles of the heart. Ventricular septal defe...

Over time, increased blood flow can damage the small blood vessels in the lungs. This causes high blood pressure in the lungs. As a result, the blood backs up and does not go to the lungs to pick up oxygen. Instead, the blood goes from the right side to the left side of the heart, allowing oxygen-poor blood to travel to the rest of the body.

Eisenmenger syndrome usually develops before a child reaches puberty. However, it also can develop in young adulthood.

Symptoms

Bluish lips, fingers, toes, and skin (cyanosis)

Chest pain

Coughing up blood

Dizziness

Fainting

Feeling tired

Shortness of breath

Stroke

Swelling in the joints caused by too much uric acid (gout)

Exams and Tests

The doctor will examine the child. During the exam, the doctor may find:

Abnormal heart rhythm (arrhythmia)

Enlarged ends of the fingersor toes (clubbing)

Heart murmur (an extra sound when listening to the heart)

The doctor will diagnose Eisenmenger syndrome by looking at the patient's history of heart problems. Tests may include:

Complete blood count (CBC)

Chest x-ray

MRI scan of the heart

Putting a thin tube in an artery to view the heart and blood vessels and measure pressures (cardiac catheterization)

Test of the electrical activity in the heart (electrocardiogram)

Ultrasound of the heart (echocardiogram)

The number of cases of this condition in the United States has dropped because doctors are now able to diagnose and correct the defect sooner, before the irreversible damage to the small lung arteries occurs.

Treatment

Older children with symptoms may have blood removed from the body (phlebotomy) to reduce the number of red blood cells, and then receive fluids to replace the lost blood (volume replacement).

Children may receive oxygen, although it is unclear whether it helps to prevent the disease from getting worse. Children with very severe symptoms may need a heart-lung transplant.

Outlook (Prognosis)

How well the infant or child does depends on whether another medical condition is present, and the age at which high blood pressure develops in the lungs. Patients with this condition can live 20 to 50 years.

Possible Complications

Bleeding (hemorrhage) in the brain

Congestive heart failure

Gout

Heart attack

Hyperviscosity (sludging of the blood because it is too thick with blood cells)

Hyperviscosity

Hyperviscosity of the newborn is the slowing and blockage of blood flow that results when there are too many red blood cells in an infant's blood....

Congenital heart defects...

Animation

Congenital heart defects - blood flow - Animation

Eisenmenger syndrome - illustration

Children born with Eisenmenger syndrome are born with a hole between the two pumping chambers -- the left and right ventricles -- of the heart (ventricular septal defect). The hole allows blood that has already picked up oxygen from the lungs to flow back into the lungs, instead of going out to the rest of the body. The increased blood flow and high pressure damages the small blood vessels in the lungs.

Eisenmenger syndrome

illustration

Congenital heart defects...

Animation

Congenital heart defects - blood flow - Animation

Eisenmenger syndrome - illustration

Children born with Eisenmenger syndrome are born with a hole between the two pumping chambers -- the left and right ventricles -- of the heart (ventricular septal defect). The hole allows blood that has already picked up oxygen from the lungs to flow back into the lungs, instead of going out to the rest of the body. The increased blood flow and high pressure damages the small blood vessels in the lungs.